Epilepsy Surgery in Children.

Epilepsy in children continues to present a major medical and economic burden on society. Left untreated, seizures can present the risk of sudden death and severe cognitive impairment. It is understood that primary care providers having concerns about abnormal movements or behaviors in children will make a prompt referral to a trusted pediatric neurologist.

Single-center experience with Beta-propeller protein-associated neurodegeneration (BPAN); expanding the phenotypic spectrum.

Beta-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA) that presents with childhood developmental delay (especially speech delay), occasionally associated with epileptic encephalopathy, autism, or Rett-like syndrome. The majority of children described to date have been severely affected, with little to no expressive speech function, severe developmental delay, and cognitive impairment. Herein, five additional patients with BPAN identified in the same center in Canada are described, four with the typical severe phenotype and one with a milder phenotype.

Generalizability of High Frequency Oscillation Evaluations in the Ripple Band.

We examined the interrater reliability and generalizability of high-frequency oscillation (HFO) visual evaluations in the ripple (80-250 Hz) band, and established a framework for the transition of HFO analysis to routine clinical care. We were interested in the interrater reliability or epoch generalizability to describe how similar the evaluations were between reviewers, and in the reviewer generalizability to represent the consistency of the internal threshold each individual reviewer. We studied 41 adult epilepsy patients (mean age: 35.

Heart rate variability in epilepsy: A potential biomarker of sudden unexpected death in epilepsy risk.

Objective: Sudden unexpected death in epilepsy (SUDEP) is a tragic and devastating event for which the underlying pathophysiology remains poorly understood; this study investigated whether abnormalities in heart rate variability (HRV) are linked to SUDEP in patients with epilepsy due to mutations in sodium channel (SCN) genes.

Methods: We retrospectively evaluated HRV in epilepsy patients using electroencephalographic studies to study the potential contribution of autonomic dysregulation to SUDEP risk. We extracted HRV data, in wakefulness and sleep, from 80 patients with drug-resistant epilepsy, including 40 patients with mutations in SCN genes and 40 control patients with non-SCN drug-resistant epilepsy.

Intravenous Immunoglobulin as a Treatment for Intractable Epilepsy Secondary to Focal Cortical Dysplasia: A Meta-analysis.

Background: The observation of a dramatic response to intravenous immunoglobulin (IVIG) by a child from our center with intractable epilepsy due to focal cortical dysplasia prompted us to perform a meta-analysis on the efficiency of IVIG in this condition. Focal cortical dysplasia is a common cause of intractable epilepsy. Microglial activation and upregulation of neuroinflammatory pathways have been documented in brain specimen from surgically treated patients with intractable epilepsy and focal cortical dysplasia.

Interrater reliability of visually evaluated high frequency oscillations.

Objective: High frequency oscillations (HFOs) and interictal epileptiform discharges (IEDs) have been shown to be markers of epileptogenic regions. However, there is currently no 'gold standard' for identifying HFOs. Accordingly, we aimed to formally characterize the interrater reliability of HFO markings to validate the current practices.

TUBA1A Mutation Associated With Eye Abnormalities in Addition to Brain Malformation.

Objective: We describe the case of a boy with a TUBA1A mutation presenting with microphthalmia and congenital cataracts in addition to microcephaly and severe brain malformation.

Methods: A boy presented in early infancy with microphthalmia, congenital cataracts, and microcephaly. His neurological course included severe hypotonia and drug-resistant epilepsy.

Infraslow status epilepticus: A new form of subclinical status epilepticus recorded in a child with Sturge-Weber syndrome.

Background: Analysis of infraslow EEG activity (ISA) has shown potential in the evaluation of patients with epilepsy and in the differentiation between focal and generalized epilepsies. Infraslow EEG activity analysis may also provide insights into the pathophysiology of refractory clinical and subclinical status epilepticus. The purpose of this report is to describe a girl with Sturge-Weber syndrome (SWS) who presented with a 96-h refractory encephalopathy and nonischemic hemiparesis and who was identified to have infraslow status epilepticus (ISSE), which successfully resolved after midazolam administration.

Efficacy of intravenous immunoglobulin in a cohort of children with drug-resistant epilepsy.

Objectives: We describe the experience of a pediatric epilepsy center regarding the efficacy of intravenous immunoglobulin for drug-resistant seizures in children.

Methods: A retrospective chart review of all children in a community-based, children's hospital neurology clinic from 2006 to 2012, inclusive, with intractable epilepsy who were treated with intravenous immunoglobulin for a minimum of six cycles was performed. Data collected included patient demographics, seizure and epilepsy syndrome type, presumed etiology for the seizures, and seizure frequency.

Infraslow EEG changes in infantile spasms.

Purpose: Infantile spasms (IS) are a devastating epileptic encephalopathy syndrome of infancy. Analysis of infraslow EEG activity (ISA) has shown potential in the presurgical evaluation of patients with epilepsy and in differentiating between focal and generalized epilepsy syndromes. Infraslow EEG activity analysis may provide insights into the pathophysiology of some difficult-to-treat epilepsy syndromes, such as IS.

Outcomes trajectories in children with epilepsy: hypotheses and methodology of a Canadian longitudinal observational study.

Background: The impact of childhood epilepsy can only be appreciated by understanding that epilepsy comprises a set of complex neurobehavioral conditions with significant social consequences, and not simply disorders of recurrent seizures. Our objective is to describe the hypotheses and methodology behind a large prospective longitudinal study that is based on a conceptual framework for understanding health outcomes. The study will quantify the specific influences--direct, mediating or moderating--that various epilepsy, comorbid, child, and family variables exert on health over the early life course.

Levetiracetam for benign epilepsy of childhood with centrotemporal spikes-three cases.

Benign epilepsy with centrotemporal spikes (BECTS), also known as benign rolandic epilepsy (BRE) of childhood represents 15% of all childhood epilepsies [Handbook of Epilepsy Treatment (2000)]. A majority of these patients do no require treatment; however, in those cases where treatment is justified, the most efficacious medication with a benign safety profile should be selected. We present three clinical cases of otherwise healthy children with BECTS who were treated only with levetiracetam.